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Prosopagnosia: a Clinical, Psychological, and Anatomical Study of Three Patients

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Prosopagnosia: a Clinical, Psychological, and Anatomical Study of Three Patients J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.40.4.395 on 1 April 1977. Downloaded from Journal ofNeurology, Neurosurgery, and Psychiatry, 1977, 40, 395-403 Prosopagnosia: a clinical, psychological, and anatomical study of three patients A. M. WHITELEY' AND ELIZABETH K. WARRINGTON From the Department ofNeurology, The London Hospital, and the Department ofPsychology, National Hospital, Queen Square, London SUMMARY Three patients with prosopagnosia are described of whom two had right occipital lesions. An analysis of visual and perceptual functions demonstrated a defect in perceptual classi- fication which appeared to be stimulus-specific. A special mechanism for facial recognition is postu- lated, and the importance of the right sided posterior lesion is stressed. Prosopagnosia is a rare but interesting condition unreliably, as pointers to cerebral lesions, and most in which recognition of faces is impaired. The cases have a left homonymous defect indicating right sufferer is quite unable to identify people purely by hemisphere disease, but not excluding a left sided their facial appearance but can do so without lesion (Meadows, 1974a). There are many cases, Protected by copyright. difficulty by their voice and by visual clues such as however, with bilateral field defects indicating clothing, hair colour, and gait. Recognition of other bilateral lesions, but there are cases with right visual material can be intact, but in some cases highly homonymous defects and cases with no field defects discriminative visual skills, such as species of birds at all. There are several case reports where surgery and types of fruit, are impaired (Bornstein, 1963; to right temporal and occipital lobes is responsible, De Renzi et al., 1968). There are often associated and a purely unilateral lesion is clinically suspected disturbances such as metamorphopsia (Critchley, (Hecaen and Angelergues, 1962; Lhermitte and 1953), achromatopsia (Meadows, 1974b), visual Pillon, 1975). There is one case of a left temporal field defects, topographical disorientation, dis- lobectomy causing prosopagnosia in a left handed turbances of body schema, constructional apraxia, patient (Tzavaras et al., 1973). In summary, it would and dressing apraxia (Hecaen and Angelergues, be agreed that a right occipito-temporal lesion is 1962). critical but the necessity of an additional left hemi- One of the outstanding questions of this condition sphere lesion, whether symmetrical or not, is still is whether a purely unilateral cerebral lesion can be questioned. responsible, or whether bilateral lesions are necessary. Another problem of prosopagnosia is the exact http://jnnp.bmj.com/ Postmortem studies, which are infrequent, give the nature of the psychological dysfunction. There are most accurate information, and the published cases few cases which have been studied in detail, and the show a common lesion in the right inferior occipito- results suggested three hypotheses. One hypothesis temporal region in the lingual and fusiform gyri. is that there is a general impairment of visuo-spatial These cases, however, also have a left hemisphere perception, and that prosopagnosia is merely one lesion which, in all but two cases, is symmetrically feature of this (De Renzi et al., 1968; Lhermitte and placed in the left occipito-temporal region (Meadows, Pillon, 1975). The second possibility is that the face 1974a; Cohn et al., 1974). In the two exceptions, the is satisfactorily perceived but it cannot be matched on September 30, 2021 by guest. left sided lesions are, respectively, a superficial to a memory store of faces (Benton and van Allen, gliosis in the parietal region (Pevzner et al., 1962), 1972). The third hypothesis is that facial perception and a tumour invading through the corpus callosum is mediated by a special perceptual process, and to the ventricular wall (Hecaen et al., 1957). The prosopagnosia is a specific defect of this system significance of these second lesions is disputed. (Tzavaras et al., 1970, 1973). It is always possible, Visual field defects can be used, although somewhat however, that there may not be a unitary explanation ofprosopagnosia and that all three factors contribute 'Present address: National Hospital, Queen Square, London WCI. to the genesis of impaired facial recognition. Accepted 12 November 1976 Further evidence for the anatomical correlates and 395 J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.40.4.395 on 1 April 1977. Downloaded from 396 A. M. Whiteley and Elizabeth K. Warrington functional deficits of prosopagnosia can be obtained Examination from studies of groups of patients having known The visual acuity was R = 6/12, L = 6/6. The optic cerebral lesions but no clinically obvious prosopag- discs were normal apart from a sheath of medullated nosia. All studies are consistent in indicating that the fibres in the right eye. There was enlargement of the right hemisphere alone is responsible for visual physiological blind spot in the right eye and a peri- perceptual skills including facial recognition, and the pheral nasal field defect on the left (Fig. 1). There was left hemisphere plays little part (Benton and van a mild left hemiparesis with an extensor plantar Allen, 1968; De Renzi et al., 1968; Tzavaras et al., response. 1970; Warrington and James, 1967a). The analysis On the ward he was completely unable to recognise of prosopagnosia derived from group studies follows the medical staff by their facial appearance but could similar lines to that proposed for single case studies. do so when they spoke. He could name all objects Patients with a right hemisphere lesion show a presented to him but identified only three of 24 Ishi- general impairment of visual discrimination for hara colour plates. He could read, write, and draw objects, shapes, and letters (Warrington and James, normally, and there was no topographical confusion, 1967b; Warrington and Taylor, 1973). There also dressing difficulty, or dysphasia. appears to be a defect in memory for faces which is Apart from hypertension (BP 170/100 mmHg), the independent ofperception (Milner, 1968; Warrington general examination was normal. and James, 1967a), and a specific defect in facial recognition when compared to recognition of other Investigations visual stimuli (Yin, 1970; Tzavaras et al., 1970). Routine investigations were normal, apart from In this paper these points are raised in the discussion raised serum cholesterol and triglycerides. The radio- of three patients with prosopagnosia. Localisation of isotope brain scan was normal, but an EEG showed the cerebral lesions was obtained and the importance mild bilateral abnormalities of background activity of the right occipito-temporal region stressed. with clear-cut episodic theta and sharp waves over theProtected by copyright. Detailed psychological assessment was undertaken left hemisphere. An EMI scan showed bilateral where the perception of faces was compared with occipital lobe infarctions, the left larger than the perception of other visual stimuli. right (Fig. 2). Case reports Progress His hypertension was treated with diuretics and CASE 1 (LH 733564) methyldopa. His symptoms remained unchanged at In March 1975, F.W., a 65 year old right handed, follow-up nine months later. retired business man, suddenly developed a transient, left sided headache followed by a mild right sided CASE 2 (LH 729447) weakness and speech difficulty. These symptoms Q.L. (a female, age 55 years, right handed, school resolved in the subsequent six weeks. teacher) presented in November 1975 with inability In June 1975 he again developed a sudden short- to recognise faces. Her symptoms began six weeks previously during an evening meal when she suddenly lived headache and visual disturbance which he http://jnnp.bmj.com/ described as 'vision going but not being blind, as noticed that 'things did not look the same'. She had though in a dense fog with everything black and no headache but felt vaguely unwell and went to bed. white'. His vision improved over the next few days The next day she noticed that she could not recognise but he could not recognise people, including his wife anyone, including her family, but could deduce who and children, by their facial appearance although he they were by their clothes, and could recognise their could do so by their voices. He described faces as voices. She said people looked younger, with their being 'halfcaste, not white, not black'. Initially he had wrinkles ironed out, and she even noted that her own reflection in a mirror was unfamiliar. She also difficulty in distinguishing such things as flowers from on September 30, 2021 by guest. foliage but this had improved by the time of admis- reported that she could not tell if the bacon was sion in January 1976. Colour vision was also disturbed cooked properly or if the potatoes were completely in that his colour television appeared black and white, peeled. She had no difficulty in finding her where- and traffic lights appeared white, but the colours of abouts or in dressing. solid objects appeared normal. He also described an interlacing pattern in his upper visual fields like dark Examination strands of rope over his eyes. He had no difficulty in Her visual acuity was R = 6/9, L = 6/9. Static peri- recognising his surroundings or in dressing. The metry disclosed an incongruent left homonymous previous medical history was unremarkable. hemianopia with an upper temporal scotoma in the J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.40.4.395 on 1 April 1977. Downloaded from Prosopagnosia: a clinical, psychological, and anatomical study ofthree patients 397 .60 *60 LEFT% RIGHT 1!/2 . 0 o so. Fig. I Visualfields in cases I and 2 charted on Goldmann perimeter. Continuous lines indicate perception ofmoving targets (4, IV, =relative intensity 1.00 and Case 1 size 16 mm2, 4, 1, =intensity 1.00 RIGHT 6/9 and size 0.25 mm2, 2, 1, = relative LEFT % R intensity 0.10 and size 0.25 mm2). Dotted area (case 2) indicates static object (4, I,) notperceived.
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